Craniosynostosis, the premature fusion of one or more of the brain’s sutures, is estimated to occur in as many as one in 2500 births.1 Fusion of the sutures typically occurs before birth or very soon thereafter. Some cases of craniosynostosis can be attributed to a genetic cause (“syndromic craniosynostosis”) but most cases are nonsyndromic. Nonsyndromic craniosynostosis can involve one or multiple sutures.

Early detection of craniosynostosis is crucial for prompt referral to tertiary care centers with experience in treating the condition and for better outcomes. Detection can be challenging as the increased prevalence of purely cosmetic head shape deformities, such as those due to positional pressure, can be mistaken for craniosynostosis and vice versa.2 For example, the recommendation for infants to sleep on their back to avoid SIDS has led to a sharp increase in positional head deformities that can be mistaken for a lambdoid suture synostosis. Unlike craniosynostosis, positional head shape deformities typically improve with time and as the child becomes ambulatory.

Guidelines suggest that physicians refer suspected cases of craniosynostosis to a specialist for evaluation based on characteristic morphological changes and not on advanced imaging (the overuse of which has been demonstrated) to minimize radiation risk to the child.1 This is particularly important as specialists can many times distinguish between true craniosynostosis and positional head deformities through examination and history, sparing the latter group unnecessary exposure to radiation. If the specialist suspects craniosynostosis, then computed tomography can be obtained to confirm the diagnosis.

In severe cases of nonsyndromal craniosynostosis, the premature closure of one or more sutures of the brain threatens to impede brain growth, to increase intracranial pressure, and to lead to head shape abnormalities as the brain, blocked by the fused suture, grows into other areas of the skull. In these cases, surgical treatment is indicated.1

Surgery for craniosynostosis is sometimes performed as a two-stage operation, though timing of surgery to correct craniosynostosis remains controversial.3 A first operation can be done early if there is increased intracranial pressure to open the sutures and relieve the pressure, after which infants may wear helmets to encourage more normal skull growth. After the child is older (usually around a year old) and has had time to build more bone in the skull (which may be needed in the reconstruction), a second operation is performed to correct any remaining deformity.

To obtain enough bone to perform the reconstruction without resorting to synthetic products, surgeons often use “split thickness grafts,” in which a portion of the patient’s own skull is split into two pieces of thinner bone to be used for filling gaps.4 These “split-thickness bone grafts” are one of the reasons the second stage of surgery is timed to occur when the child is older, when the bone is sufficiently thick to split.

Craniofacial surgery can be associated with large amounts of blood loss and the need for multiple transfusions, which can lead to serious adverse effects such as TRALI.1 Appropriate steps should be taken to minimize blood loss and the need for transfusion, such as careful dissection of the scalp layer by layer, with an electrocautery device used to close off small vessels. Planning surgery using 3D models of the patient’s skull and eventually practicing on those skulls before the surgery can reduce operative time, blood loss, and the need for transfusion.

“One of these reasons these models are so wonderful to have before surgery is that you can look at the model of the patient’s skull, measure it, and even cut it and you can essentially plan the entire operation before walking into the operating room,” said MUSC Children's Health pediatric neurosurgeon Ramin Eskandari, M.D., who performs these surgeries to correct craniosynostosis in collaboration with MUSC Health craniofacial surgeon Jason P. Ulm, M.D.

Join Eskandari and Ulm on September 22 at 6 pm EST on the free case-sharing app Figure 1 (available on iOS and Android; http://figure1.com) for an MUSC Health Virtual Grand Rounds on surgical correction of nonsyndromal craniosynostosis.